Chronic liver disease is a major complication of cystic fibrosis. Its incidence and severity show marked heterogeneity, even among the homogeneous group of homozygous DeltaF508 patients, suggesting that environmental or genetic factors other than the deletion DeltaF508 may influence the development of cystic fibrosis related liver disease. We investigated whether the allelic variants of mannose...

OBJECTIVE The aim of this study was to determine relative contributions of transplacental flux vs. fetal production for inositol and mannose in normal term pregnancies. STUDY DESIGN Seven term uncomplicated pregnancies undergoing cesarean section were infused with (13)C- and (2)H-labeled isotopes of glucose, inositol, and mannose until a steady state was achieved. Maternal and fetal concentra...

The macrophage mannose receptor is an integral membrane protein expressed on the surface of tissue macrophages. After ligation of mannose-rich glycoconjugates or pathogens, the receptor mediates endocytosis and phagocytosis of the bound ligands by macrophages. The cDNA-derived primary structure of the mannose receptor predicts a cysteine-rich NH2-terminal domain, followed by a fibronectin type ...

Recycling of mannose 6-phosphate receptors was investigated by microinjection of F(ab) fragments against their carboxy-terminal peptides (residues 54-67 or 150-164 of the cytoplasmic domain of 46 kDa and 300 kDa mannose 6-phosphate receptor, respectively). For each receptor, masking the carboxy-terminal peptide by the corresponding F(ab) fragments resulted in complete depletion of the intracell...

A receptor which recognizes glycoproteins bearing terminal mannose residues has been isolated from human placental membranes. Washed membranes were solubilized with buffer containing 1% Triton X-100 and applied to a mannose-Sepharose affinity column. The column was eluted with buffer containing 200 mM mannose and 1% cholate. The major protein eluted exhibited a molecular weight of 175 kDa on so...

Mycobacteria are the causative agents of tuberculosis and several other significant diseases in humans. All species of mycobacteria synthesize abundant cell-wall mannolipids (phosphatidylinositol mannosides, lipoarabinomannan), a cytoplasmic methylmannose polysaccharide and O-mannosylated glycoproteins. To investigate whether these molecules are essential for mycobacterial growth, we have gener...

Mannose-binding proteins have been isolated from the liver of rats and humans and subsequently been found in the serum of rats, rabbits, and humans. We report the isolation of cDNA clones isolated from a human liver cDNA library that encodes a human mannose-binding protein. The primary structure has three domains: (a) an NH2-terminal cysteine-rich segment of 19 amino acids which appears to be i...

The effects of N-linked-oligosaccharide-processing inhibitors on the fusion of rat L6 myoblasts to form myotubes were examined. The glucosidase inhibitor N-methyl-1-deoxynojirimycin (MDJN) greatly inhibited fusion, whereas the mannosidase inhibitor 1-deoxymannojirimycin (ManDJN) had relatively little effect, although both compounds prevented the formation of N-linked complex oligosaccharides. T...

A comparison of the rotation of two pairs of sugars, which can not form a 1 4 ring shows that one pair gives an epimeric difference in rotation, which agrees with the value obtained from a-d-mannose (+ 30) and the normal form of glucot? while the other pair gives a value which agrees with that obtained from ^ESZJnose (-17) and the normal form of glucose. These results may be interpret ,.,1 as i...